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Retro-Odontoid Pseudotumor Formation in the Context of Various Acquired and Congenital Pathologies of the Craniovertebral Junction and Surgical Techniques.

ABSTRACT: Retro-odontoid pseudotumor formation consists of an abnormal growth of granulation tissue typically posterior to the odontoid process, resulting as a manifestation of atlantoaxial instability. This instability can occur as a result of conditions ranging from severe mechanical trauma to metabolic disease or autoimmune conditions such as rheumatoid arthritis. A pseudotumor may impinge on the spinal nerves or even the spinal cord and brainstem, manifesting symptoms from severe neck pain to cervicomedullary compression or myelopathy, and in some cases even sudden death. The objective of this review is to consolidate the findings in published case reports and relevant prior literature reviews regarding the formation of retro-odontoid pseudotumor. We address the pathophysiology involved in acquired and congenital pseudotumor formation, including those associated with rheumatoid arthritis (panni). Additionally, we discuss past and current operative techniques designed to curtail and ultimately regress a retro-odontoid pseudotumor and pannus. Surgical techniques that are addressed include ventral decompression (both transoral and transnasal), dorsal decompression, and indications for posterior instrumentation in pannus formation, particularly in cases that may be sufficiently treated in lieu of an anterior approach. Finally, we will examine the role of external orthoses as both a method of conservative treatment as well as a potential adjunct to the aforementioned surgical procedures.

SUBMITTER: Fiani B

PROVIDER: S-EPMC8021814 | biostudies-literature |

REPOSITORIES: biostudies-literature

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Full-Endoscopic Transcervical Ventral Decompression for Pathologies of Craniovertebral Junction: Case Series.

Project description:Odontoidectomy is very effective for the decompression of the ventral craniovertebral junction (CVJ). Various approaches are available for the direct ventral decompression of the CVJ. Because there are many disadvantages of open transoral approach, endoscopic odontoidectomy was developed. There are 3 approaches in endoscopic odontoidectomy. We report transcervical retropharyngeal endoscopic approach for the ventral CVJ in this paper. Three patients with different pathologies received operations using this approach. The decompression was enough and surgical invasion was less in all patients. Each endoscopic approach has some advantages and different working regions due to their approach trajectories, but transcervical retropharyngeal approach is very familiar for our neurospinal surgeons and has a relatively large working area. This approach might have the chance to take the place of open transoral approach for endoscopic spinal surgeons.

| S-EPMC7410384 | biostudies-literature

Surgery for craniovertebral junction pathologies: minimally invasive anterior submandibular retropharyngeal key-hole approach.

Project description:BackgroundOur objective was to develop a new, minimally invasive surgical technique for the resolution of craniovertebral junction pathologies, which can eliminate the complications of the previous methods, like liquor-leakage, velopharyngeal insufficiency and wound-dehiscence associated with the transoral or lateral approaches.MethodsDuring the first stage of the operation, three patients underwent occipito-cervical dorsal fusion, while the fourth patient received C1-C2 fusion according to Harms. C1-C2 decompressive laminectomy was performed in all four cases. Ventral C1-C2 decompression with microscope assisted minimally invasive anterior submandibular retropharyngeal key-hole approach (MIS ASR) method was performed in the second stage. The MIS ASR-similarly to the traditional anterior retropharyngeal surgery-preserves the hard and soft palates, yet can be performed through a 25 mm wide incision with the use of only one retractor.ResultsThe MIS ASR approach was a success in all four cases, there were no intra- and postoperative complications. This method, compared to the transoral approach, provided on average 23% (4.56 cm2/6.05 cm2) smaller dural decompression area; nonetheless, the entire pathology could be removed in all cases. After the surgery, all patients have shown significant neurological improvement.ConclusionBased on the outcome of these four cases we think that the MIS ASR approach is a safe alternative to the traditional methods while improving patient safety by reducing the risk of complications.

| S-EPMC8054389 | biostudies-literature

Evaluation and Surgical Planning for Craniovertebral Junction Deformity.

Project description:Craniovertebral junction (CVJ) deformity is a challenging pathology that can result in progressive deformity, myelopathy, severe neck pain, and functional disability, such as difficulty swallowing. Surgical management of CVJ deformity is complex for anatomical reasons; given the discreet relationships involved in the surrounding neurovascular structures and intricate biochemical issues, access to this region is relatively difficult. Evaluation of the reducibility, CVJ alignment, and direction of the mechanical compression may determine surgical strategy. If CVJ deformity is reducible, posterior in situ fixation may be a viable solution. If the deformity is rigid and the C1-2 facet is fixed, osteotomy may be necessary to make the C1-2 facet joint reducible. C1-2 facet release with vertical reduction technique could be useful, especially when the C1-2 facet joint is the primary pathology of CVJ kyphotic deformity or basilar invagination. The indications for transoral surgery are becoming as narrow as a treatment for CVJ deformity. In this article, we will discuss CVJ alignment and various strategies for the management of CVJ deformity and possible ways to prevent complications and improve surgical outcomes.

| S-EPMC7538356 | biostudies-literature

Suture Repair in Endoscopic Surgery for Craniovertebral Junction.

Project description:OBJECTIVE:Endoscopic approaches to the craniovertebral junction (CVJ) have been established as viable and effective surgical treatments in the past decade. One of the major complications is leakage of the cerebrospinal fluid (CSF). This study aimed to investigate the efficacy and feasibility of suture closure at the nasopharyngeal mucosa upon durotomy. METHODS:A series of consecutive patients who underwent different endoscopic approaches to the CVJ were retrospectively reviewed. The pathologies, surgical corridors, neurological and functional outcomes, radiological evaluations, and complications were analyzed. Different strategies of repair for the intraoperative CSF leakage were described and compared. RESULTS:A total of 22 patients covering 13 years were analyzed. There were 12, 2, and 8 patients who underwent transnasal, transoral, and combined approaches, respectively. There were 8 patients (36.4%) who experienced intraoperative CSF leakage, and were grouped into 2: 4 in the nonsuture (NS) group and 4 in the suture-repaired (SR) group. The NS group had 3 (75%) persistent CSF leakages postoperation that caused 1 mortality, whereas patients of the SR group had only 1 minor CSF rhinorrhea that healed spontaneously within days. CONCLUSION:In this series of 22 patients who required anterior endoscopic resection of pathologies at the CVJ, there was 1 (4.5%) serious complication related to CSF leakage. For patients who had no durotomy, the mucosal incision at the nasopharynx usually healed rapidly and there were few procedure-related complications. For patients with intraoperative CSF leakage, suture closure was technically challenging but could significantly lower the risks of postoperative complications.

| S-EPMC6603818 | biostudies-literature

Surgical Management of Craniovertebral Junction Schwannomas: A Systematic Review.

Project description:BackgroundCraniovertebral junction (CVJ) schwannomas are rare, with surgery and stereotactic radiosurgery (SRS) being effective yet challenging options. We systematically reviewed the literature on CVJ schwannomas.MethodsPubMed, Scopus, Web-of-Science, and Cochrane were searched following the PRISMA statement to include studies reporting CVJ schwannomas. Clinical features, management, and outcomes were analyzed.ResultsWe collected 353 patients from 101 included articles. Presenting symptoms were mostly neck pain (30.3%) and headache (26.3%), with most cranial neuropathies involving the XII (31.2%) and X (24.4%) nerves. Most tumors originated from C2 (30.9%) and XII (29.4%) nerves, being extracranial (45.1%) and intradural-extradural (44.2%). Erosion of C1-C2 vertebrae (37.1%), the hypoglossal canal (28.3%), and/or jugular foramen (20.1%) were noted. All tumors were operated, preferably with the retrosigmoid approach (36.5%), with the far-lateral approach (29.7%) or with the posterior approach and cervical laminectomy (26.9%), far-lateral approaches (14.2%), or suboccipital craniotomy with concurrent cervical laminectomy (14.2%). Complete tumor resection was obtained most frequently (61.5%). Adjuvant post-surgery stereotactic radiosurgery was delivered in 5.9% patients. Median follow-up was 27 months (range, 12-252). Symptom improvement was noted in 88.1% of cases, and cranial neuropathies showed improvement in 10.2%. Post-surgical complications occurred in 83 patients (23.5%), mostly dysphagia (7.4%), new cranial neuropathies (6.2%), and cerebrospinal fluid leak (5.9%). A total of 16 patients (4.5%) had tumor recurrence and 7 died (2%), with median overall survival of 2.7 months (range, 0.1-252).ConclusionsMicrosurgical resection is safe and effective for CVJ schwannomas. Data on SRS efficacy and indications are still lacking, and its role deserves further evaluation.

| S-EPMC9319499 | biostudies-literature

Retro-odontoid calcium pyrophosphate dehydrate deposition: surgical management and review of the literature.

Project description:Study Design Case report and review of the literature. Objective A retro-odontoid mass is a rare cause of cervical compression and myelopathy. The differential diagnosis includes the following: metastatic disease, primary tumor, collagen disorder, or inflammatory disease. Calcium pyrophosphate dihydrate (CPPD) deposition has been referred to as "crowned dens syndrome" when there are periodontoideal calcifications. There are only a few reported cases where CPPD presents as a cystic retro-odontoid mass in the atlanto-dens interval. In previous descriptions of surgical intervention, transoral resection of the mass is associated with significant morbidity and usually requires stabilization. The objective of this article is to report a case of an unusual presentation of CPPD disease of C1/C2, where we used a novel, minimally invasive surgical technique for decompression without fusion. Patients and Methods An 83-year-old female patient presented with progressive cervical myelopathy over a 3-month period. Computed tomography and magnetic resonance imaging demonstrated a cystic odontoid mass with a separate retro-odontoid compressive mass. A novel, minimally invasive transoral aspiration was performed. Histologic confirmation of CPPD was obtained. Results Postop imaging showed satisfactory decompression, which was maintained at the 6-month follow-up. This correlated with clinical improvement postop and 6-month follow-up. Conclusion CPPD in the atlanto-dens interval may present as a cystic retro-odontoideal mass and should be included in the differential. We used a transoral minimally invasive approach to aspirate the cyst. This novel technique avoided the need for a stabilization procedure or morbid transoral resection and provided excellent results immediately and at 6 months.

| S-EPMC3969430 | biostudies-other

Management of post-traumatic craniovertebral junction dislocation: A PRISMA-compliant systematic review and meta-analysis of casereports.

Project description:Although historically considered fatal, with the advent of improved pre-hospital care, traumatic dislocation of the craniovertebral junction (CVJ) has been increasing in prevalence in neurosurgical centers. As more survivors are reported each year, a timely review with meta-analysis of their management seems necessary. PRISMA checklist was followed step by step. PubMed and Web of Science databases were searched using words "craniovertebral junction dislocation" and their corresponding synonyms. Study eligibility criteria included research studies from 2015 onwards that delineated adult and pediatric patients with confirmed post-traumatic atlantooccipital dislocation (AOD) or atlantoaxial dislocation (AAD) who survived until proper treatment. Of 1475 initial records, 46 articles met eligibility criteria with a total of 141 patients with traumatic CVJ dislocation. Of the patients, 90 were male (63.8%). Mean age of the cohort was 33.3 years (range 1-99 years). Trauma that most often led to this injury was road traffic accident (70.9%) followed by falls (24.6%). The majority of authors support posterior instrumentation of C1-C2 (45.2%) especially by means of Goel-Harms method. At mean follow-up of 15.4 months (range 0.5-60 months), 27.2% of treated patients remained neurologically intact. Of initially symptomatic, 59% improved, 37% were stable, and 4% deteriorated. Instrumenting the occiput in cases of pure AAD was associated with lower chance of neurological improvement in chi-square test (p = 0.0013) as well as in multiple linear regression (β = - 0.3; p = 0.023). The Goel-Harms C1-C2 fusion is currently the most frequently employed treatment. Many survivors remain with no deficits or improve, rarely deteriorate. Involving the occiput in stabilization in cases of AAD without AOD might be related with worse neurological prognosis.

| S-EPMC8121741 | biostudies-literature

Knockdown of best1 Gene in Zebrafish Caused Abnormal Neuronal and Skeletal Development - A Subtype of Craniovertebral Junction Malformation?

Project description:ObjectiveTo investigate the developmental defects caused by knockdown of best1 gene in zebrafish as a model for a subtype of craniovertebral junction (CVJ) malformation.MethodsTwo antisense morpholinos (MOs) were designed targeting zebrafish best1 to block translation (ATG-MO) or to disrupt splicing (I3E4-MO). MOs were microinjected into fertilized one-cell embryos. Efficacy of splicing MO was confirmed by reverse transcription-polymerase chain reaction. Phenotypes were analyzed and quantified by microscopy at multiple developmental stages. Neuronal outgrowth was assessed in transgenic zebrafish expressing green fluorescent protein in neurons. Skeletal ossification was visualized by Calcein staining.ResultsKnockdown of best1 resulted in zebrafish embryos with shorter body length, curved axis, low survival rate, microcephaly, reduced eye size, smaller head and brain, impaired neuronal outgrowth, and reduced ossification of craniofacial and vertebral bone.ConclusionBest1 gene plays critical roles in ophthalmologic, neurological and skeletal development in zebrafish. A patient with a premature stop codon in BEST1 gene exhibited similar phenotypes, implying a subtype of CVJ malformation.

| S-EPMC11224734 | biostudies-literature

A Dual Approach for the Management of Complex Craniovertebral Junction Abnormalities: Endoscopic Endonasal Odontoidectomy and Posterior Decompression with Fusion.

Project description:Background:Ventral brainstem compression secondary to complex craniovertebral junction abnormality is an infrequent cause of neurologic deterioration in pediatric patients. However, in cases of symptomatic, irreducible ventral compression, 360° decompression of the brainstem supported by posterior stabilization may provide the best opportunity for improvement in symptoms. More recently, the endoscopic endonasal corridor has been proposed as an alternative method of odontoidectomy associated with less morbidity. We report the largest single case series of pediatric patients using this dual-intervention surgical technique. The purpose of this study was to evaluate the surgical outcomes of pediatric patients who underwent posterior occipitocervical decompression and instrumentation followed by endoscopic endonasal odontoidectomy performed to relieve neurologic impingement involving the ventral brainstem and craniocervical junction. Methods:Between January 2011 and February 2017, 7 patients underwent posterior instrumented fusion followed by endonasal endoscopic odontoidectomy at our unit. Standardized clinical and radiological parameters were assessed before and after surgery. A univariate analysis was performed to assess clinical and radiologic improvement after surgery. Results:A total of 14 operations were performed on 7 pediatric patients. One patient had Ehlers-Danlos syndrome, 1 patient had a Chiari 1 malformation, and the remaining 5 patients had Chiari 1.5 malformations. Average extubation day was postoperative day 0.9. Average day of initiation of postoperative feeds was postoperative day 1.0. Conclusions:The combined endoscopic endonasal odontoidectomy and posterior decompression and fusion for complex craniovertebral compression is a safe and effective procedure that appears to be well tolerated in the pediatric population.

| S-EPMC6580888 | biostudies-literature

Transoral approach and extended modifications for lesions of the ventral foramen magnum and craniovertebral junction.

Project description:OBJECTIVES: To describe our method of performing the transoral approach and the extended approaches to the ventral foramen magnum and craniovertebral junction and review the technical aspects and operative nuances. DESIGN: Review. RESULTS: The transoral approach provides direct midline exposure to access extradural disease located at the craniovertebral junction and ventral foramen magnum. The corridor of exposure is generally limited by the extent to which the patient can open his or her mouth. The location of the hard palate relative to the craniovertebral junction limits superior exposure, whereas the mandible and base of the tongue limit the inferior exposure. In most cases, exposure can be obtained from the inferior clivus to the middle to lower C2 vertebral body. Extended transoral approaches can be performed to increase exposure if necessary. These approaches include transmaxillary (Le Fort I maxillotomy), transmaxillary with a midline palatal split (extended "open-door" maxillotomy), transpalatal, and median labiomandibular glossotomy (transmandibular split). CONCLUSIONS: The transoral approach effectively provides direct access to extradural midline lesions of the craniovertebral junction. A specialized retractor system can expose the inferior clivus to the C2 body. Extended approaches as described can access lesions that extend beyond these limits.

| S-EPMC2459326 | biostudies-other

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